General Measures

Supportive:

• Although surgical intervention has become the medical standard, supportive measures are sometimes offered, especially when multiple noncardiac congenital anomalies exist or when severe multiorgan system damage is present.
• The preoperative goal is to balance the systemic and pulmonary circulations provided by the RV to a Qp/Qs (ratio of pulmonary to systemic blood flow) of ~1:1, usually achieved with a pulse oximetry measurement of 75%.
• Prostaglandin E1 infusion: 0.05–0.1 mcg/kg/min
• Aggressive treatment of metabolic acidosis with fluid boluses, bicarbonate, and/or tromethamine (THAM).
• 0.21 FiO₂, goal PaO₂ of 35–40 mm Hg
• Careful use of small amounts of inotropic agents (in cases of sepsis or RV failure). Aggressive use of inotropic agents (alpha effect) may worsen systemic perfusion.